Cardiomyopathies Flashcards
Mitral doppler findings RCM
Fast E wave, stops abruptly
Almost no A wave
PV doppler RCM
Very little systolic flow
Rapid inflow in early diastole, stops abruptly
Treatment of RCM
Cautious diuresis
Cautious BB
May need transplant
Amyloid features
Very thick walls
Speckled appearance
EKG normal or low voltage
Hypereosinophilia features
Fuzzy around myocardium, thrombus-like deposition in apices
If mobile elements -> anticoagulant
Hydroxyurea, steroids
ARVD features
Fatty replacement of RV free wall -> R heart failure
Arrhythmias
Repolarization abnormality, conduction delay
30% with FH
Often need ICD, no competitive athletics
LV non-compaction features
Non-compacted myocardium
Crypts and recesses
Increased risk for LV dilation, CHF, possible SCD
LV non-compaction and normal systolic function
treat as stage B HF
Use HCM / DCM criteria for SCD risk
LV non-compaction and reduced systolic function
treat as stage B or C HF
A/C if EF < 35%
Use HCM / DCM criteria for SCD risk
Ddx for thick walls
LVH HCM Renal failure Amyloid Glycogen storage disease Anderson-fabry's disease Freiderich's ataxia
Athletic heart
LVH LVEDD > 55 mm Responds to de-training Super-normal exercise capacity No MRI scar tissue or perfusion defects
EKG in apical HCM
Deep narrow T waves
HCM echo screening in children
No later than onset of puberty or at any consideration of competitive athletics
Every 12-18 months
HCM echo screening in adults
Every 5 years
Stop at 60 if normal
Genetic testing for screening in HCM
If HCM patient has known mutation, genetic testing preferred for screening
If no known mutation, imaging necessary
Pathophysiology of HCM
Diastolic dysfunction in 100% (increased LAP, dec CO)
Obstruction, SAM / MR in 70% (high LAP, subendocardial ischemia, MR, decreased CO)
Significant gradient at rest in HCM
> 30
Gradient to produce class III-IV symptoms in HCM
> 40-50
HCM LVOT gradient worsens with
More vigorous contraction
Decreased resistance
Decreased volume
Avoid in HCM
Positive inotropes
Pure vasodilators
High-dose diuretics
Meds in HCM
Decrease contractility
Beta blockers
Verapamil, diltiazem
Disopyramide as adjunct
Indications for myectomy / alcohol septal ablation in HCM
LVOT obstruction
Symptoms and impaired QoL despite meds
Risk factors for SCD in HCM
NSVT Massive LVH FH 1st deg relative < 45 Unexplained syncope last 6 months Abnormal BP response to exercise
ICD indications for HCM class I
Prior cardiac arrest or sustained VT
ICD indications for HCM class IIa
FH-SCD, LV thickness > 30mm, recent unexplained syncope
NSVT or abnormal BP response to exercise + risk modifiers
Risk modifiers for HCM and ICD
Young Wall thickness close to 30 Remote FH SCD LVOT obstruction without planned intervention Delayed enhancement Septal ablation Known specific HCM mutations Syncope < 5 years ago
Sarcoid cMRI
Midmyocardial and epicardial LGE, particularly in basal septum and basal lateral wall
Fabry’s disease
Preserved EF, chest pain, normal coronaries, LVH, renal dysfunction, FH (X-linked)
CYP inhibitors which increase taco or calcineurin inhibitor levels
Azoles
Diltiazem
Verapamil
pHTN 6MWT target for success
> 380 m
HF predictors of poor hospital prognosis
low SBP, elevated BUN, elevated Cr
Dose for anthracycline toxicity
3-5% with 400 mg/m
7-26% at 550 mg/m
18-48% at 700 mg/m
Risk factors for antracycline toxicity
1) intravenous bolus administration,
2) higher single doses,
3) history of prior mediastinal irradiation,
4) use of other concomitant agents known to have cardiotoxic effects, such as cyclophosphamide, trastuzumab, and paclitaxel,
5) female sex,
6) underlying cardiovascular disease,
7) extremes of patient age (both very young and old age), and
8) increased length of time since anthracycline completion.
Diagnosis of familial cardiomyopathy
3 generations involved, genetic testing not necessary
Screening for familial cardiomyopathy
1st degree relatives
Echo every 3-5 years
Hereditary hemochromatosis
Iron overload (ferritin > 300, transferrin saturation >55%), T2 < 20 ms on CMR, Evidence of heart disease (reduced EF,rrestrictivefilling, pulmonary hypertension)
IV iron in HF
Class II or III HF, ferritin < 100 or ferritin 100-299 and iron sat <20%
ARVC EKG
TWI in anterior precordial leads, high-frequency, low amplitude deflection at end of QRS complex (epsilon waves)
ARVC echo
RV dilatation, RV dysfunction
ARVC mutation
AD
plakoglobin
Killip 1
No signs of hF
Killip 2
Rales (crackles) in the lungs, an S3, and elevated jugular venous pressure
Killip 3
Acute pulmonary edema
Killip 4
Cardiogenic shock or hypotension (systolic blood pressure <90 mm Hg) and evidence of peripheral vasoconstriction.
Chemotherapy induced cardiomyopathy
decrease in cardiac LVEF that was either global or more severe in the septum
HF symptoms
Decline in LVEF of ≤5-55% with accompanying signs or symptoms of CHF or a decline in LVEF of ≤10-55% without accompanying signs or symptoms.
Cardiotoxic agents to monitor
anthracyclines, trastuzumab, sunitinib
Tyrosine kinase inhibitors complications
Thrombotic events
Vent to increase O2
FiO2, PEEP
Vent to increase ventilation
RR, tidal volume
